Midterm Outcomes in Supravalvular Aortic Stenosis Demonstrate the Superiority of Multisinus Aortoplasty

Kaushal, Sunjay; Backer, Carl L.; Patel, Shivani; Gossett, Jeffrey G.; Mavroudis, Constantine

doi:10.1016/j.athoracsur.2010.02.019

Cited by 43 publications

(53 citation statements)

References 18 publications

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“…In contrast to SVAS, pulmonary artery stenosis 17,20,21 in this population often regressed or resolved with time. Wessel et al 50 reported the pressure gradient decreased spontaneously from a mean of 23 to 9.5 mmHg in 46.9% (23/49) patients with pulmonary artery stenosis over 14 years.…”

Section: Adverse Cardiac Eventsmentioning

confidence: 99%

“…Kaushal et al 20 pointed out that the surgical indications for reoperation were residual aortic stenosis, aortic insufficiency and subvalvular stenosis. In contrast, Croti et al 22 demonstrated that SVAS patients receiving the Doty or Brom technique rarely required aortic reoperations, as for their low incidence of aortic regurgitation and an expanded ascending aorta without gradient as well as good ventricular performance.…”

Section: Svasmentioning

confidence: 99%

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Congenital heart defects in Williams syndrome

Yuan¹

2017

Turk J Pediatr

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Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

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Section: Adverse Cardiac Eventsmentioning

confidence: 99%

Section: Svasmentioning

confidence: 99%

Congenital heart defects in Williams syndrome

Yuan¹

2017

Turk J Pediatr

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“…Kaushal et al concluded that the multi-sinus technique was superior to the standard single-patch technique. Kaushal's data demonstrated that the only risk factor predictive of a recurrent gradient requiring reoperation was a sub-aortic myectomy performed at the first procedure (8) . Deo et al showed that the need for reoperation did not show significant difference between the single-patch and three-sinus patch groups.…”

Section: Discussionmentioning

confidence: 99%

“…Since our patients had no WilliamsBeuren syndrome, this could be accepted as an advantage for freedom from reoperation. Metton et al reported that bicuspid aortic valve was a risk factor associated with late residual obstruction (4) , but Kaushal et al did not show that bicuspid aortic valve was a risk factor for reintervention (8) .…”

Section: Discussionmentioning

confidence: 99%

“…The mean gradient was 33 ± 18 mmHg in the single-patch group and 18 ± 12 mmHg in the three-sinus repair, both of which seem high compared with other series (9) . In spite of the opposite view of some authors, multi-sinus reconstructions (inverted bifurcated patchplasty and three-sinus reconstruction) resulted in superior haemodynamics and were associated with reductions in reoperation rate and mortality in most of the articles (8)(9)(10) . The majority of patients with Williams-Beuren syndrome-related SVAS also have pulmonary artery obstruction.…”

Section: Discussionmentioning

confidence: 99%

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The Brom Technique for Congenital Supravalvular Aortic Stenosis: A Report of Two Cases

Karaçelik¹,

Abud²,

Karagöz³

et al. 2016

Kosuyolu Heart Journal

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Various surgical techniques have been described to repair congenital supravalvular aortic stenosis, but the best technique is still controversial. The three-patch repair reconstructs a symmetric aortic root and is supposed to restore a normal physiology. We present two successfully operated patients who had congenital supravalvular aortic stenosis using symmetric three-patch repair. We will discuss the decision making of surgical techniques, and its effects of postoperative course of this pathology in this case report.

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